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A Yeast Model for Understanding ALS: Fast, Cheap, and Easy to Control

PLoS Biology
Public Library of Science
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DOI: 10.1371/journal.pbio.1001053
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pbio.1001053 1..2 Synopsis A Yeast Model for Understanding ALS: Fast, Cheap, and Easy to Control Richard Robinson* Freelance Science Writer, Sherborn, Massachusetts, United States of America Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease in which the motor neurons of the central nervous system—those cells of the brain and spinal cord that control muscles—die off. The resulting paralysis typically leads to death within 3–5 years of onset. The cause of the disease in the majority of cases is complete- ly unknown, and there is no treatment that halts or significantly slows the disease. In the United States ALS is often known as Lou Gehrig’s Disease, after the baseball player who famously suffered from it. Several genes have been linked to ALS. The most recent, called FUS (fused in sarcoma, a reference to the context of its discovery), is the subject of two new studies in this issue of PLoS Biology, one by Zhihui Sun, Zamia Diaz, James Shorter, Aaron Gitler, and colleagues, and the other by Shulin Ju, Gregory Petsko, Dagmar Ringe, and colleagues. Both explore FUS biology in yeast, and highlight the potential for mod- eling elements of complex diseases in this simplest of eukaryotic cell systems. Results from both studies suggest that defects in RNA processing and transport may be a central element of ALS pathophysiology. Within the cytoplasm of motor neurons in ALS patients, proteins aggregate to form insoluble clumps, called inclusions, which can include both FUS and another ALS- causing protein, called TDP-43. When the two research groups overexpressed human FUS in yeast, they observed cytoplasmic inclusions. Inclusions form in most of the neurodegenerative diseases, including Alz- heimer’s disease and Parkinson’s disease, suggesting that common defects in protein handling may link all of them. In humans, FUS is found predominantly in the nucleus, and at least some ALS- associated mutations

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